The inflammatory reaction pattern distinguishes dysferlinopathies from idiopathic inflammatory myopathies

Dysferlinopathies are often accompanied by inflammatory infiltrates and may even mimic idiopathic inflammatory myopathies. In the present study, the inflammatory reaction profile with respect to the cellular composition of the infiltrates and the expression of major cytokines was characterized by immunofluorescence in dysferlinopathies and compared to idiopathic inflammatory myopathies. Infiltrates consisted mainly of CD4+ CD25- T cells and macrophages. The prominent expression of interferon-gamma may underlie the marked upregulation of MHC class I antigen on the vast majority of muscle fibres. Remarkably, these…